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Skin Morphology, Development and Physiology
Published in Heather A.E. Benson, Michael S. Roberts, Vânia Rodrigues Leite-Silva, Kenneth A. Walters, Cosmetic Formulation, 2019
Kenneth A. Walters, Michael S. Roberts
Scaly skin diseases and impaired barrier function can be a consequence of a disrupted desquamation process. It has been known for some time that desquamation is associated with a conversion of cholesterol sulphate to cholesterol (Long et al., 1985) and X-linked ichthyosis, a scaly disease characterized by a disrupted desquamation process, is identified with a lack of the enzyme cholesterol sulphatase (Koppe et al., 1978). Later work by Sato et al. (1998) demonstrated that hyperkeratosis attributable to desmosomes is associated with an increased content of cholesterol sulphate in patients with X-linked ichthyosis. It is apparent that cholesterol sulphate retards desquamation by acting as a serine protease inhibitor. Mutations in the corneodesmosin gene can lead to peeling skin syndrome (Israeli et al., 2011), and mutations in the gene encoding for a serine protease inhibitor can lead to Netherton syndrome, in which atopy and premature desquamation occur (Deraison et al., 2007; Igawa et al., 2013).
Nail changes in systemic diseases and drug reactions
Published in Eckart Haneke, Histopathology of the NailOnychopathology, 2017
In the early stage, nummular eczema (dermatitis) has to be considered. The epidermal hyperplasia may lead to the misdiagnosis of psoriasis. Epidermal pallor, keratinocyte necrosis, and cleft formation are also characteristic for other types of necrolytic erythemas, such as seen in the glucagonoma syndrome, pellagra, or zinc deficiency.108 A high degree of suspicion is necessary to make the correct diagnosis.109 Acral peeling skin syndrome shows a lamellar exfoliation of the horny layer of the normal epidermis without an inflammatory infiltrate.110
Advances in understanding of Netherton syndrome and therapeutic implications
Published in Expert Opinion on Orphan Drugs, 2020
Evgeniya Petrova, Alain Hovnanian
Differential diagnoses include peeling skin syndrome, Omenn syndrome and other primary immune deficiency syndromes, hyper IgE syndromes, severe atopic dermatitis and severe skin dermatitis, multiple allergies and metabolic wasting (SAM) syndrome.